A rare case of simultaneous occurrence of non-arteritic anterior ischemic optic neuropathy and corpus callosum ischemia.

PURPOSE: To report a case of non-arteritic anterior ischemic optic neuropathy (NAION) in an elderly patient with ischemia of the left splenium of the corpus callosum, providing details of the diagnostic work-up and subsequent follow-up. METHODS SECTION: Case report. RESULTS: A pseudophakic 80 years-old woman referred complaining sudden visual impairment in the left eye (LE) in concomitance with episode of hypertensive crisis. Fundus examination showed diffuse swelling of optic disc associated with flame peripapillary hemorrhages in LE and small crowded disc in right eye (RE). A superior altitudinal defect with arcuate defect including the blind spot were detected at the visual field in the LE. The patient was diagnosed with NAION. Five days later the patient complained a further vision loss and a pathological area within the left splenium of corpus callosum, consistent ischemia, was depicted at magnetic resonance imaging of brain. Corpus callosum infarction was completely asymptomatic and neurological evaluation was normal. At 45 days follow-up fundus examination showed white ischemic nerve while visual field was irreversibly constricted with tubular defect in LE. CONCLUSION: In case of NAION linked with corpus callosum ischemia multimodal imaging and systemic work-up play a pivotal role for an early diagnosis.

Comparison of central corneal thickness measured by automatic and manual analysis of optical coherence tomography.

PURPOSE: To compare central corneal thickness (CCT) measured with optical coherence tomography (OCT) using an automatic algorithm (A-OCT) vs. manual measurements (M-OCT) with respect to the gold standard ultrasound pachymetry (USP). METHODS: CCT measurements were performed on both eyes of 28 healthy subjects at four times of the day. A-OCT used an automatic software analysis of the corneal image, M-OCT was performed by two operators by setting a digital calliper on the corneal borders, and USP was performed after corneal anesthesia. Measurements were compared using a three-way repeated measures ANOVA. Bland-Altman plots were used to evaluate the agreement between OCT measurements and USP. RESULTS: Both A-OCT and M-OCT significantly underestimated the USP measures, with the mean difference, i.e., the systematic error, being larger for A-OCT (- 19.0 µm) than for M-OCT (- 6.5 µm). Good reproducibility between the two operators was observed. Bland-Altman plots showed that both OCT methods suffered from proportional errors, which were not affected by time and eye. CONCLUSIONS: Measuring CCT with OCT yielded lower values than USP. Therefore, clinicians should be aware that corneal thickness values may be influenced by the measurement method and that the various devices should not be used interchangeably in following up a given patient. Intriguingly, M-OCT had less systematic error than A-OCT, an important outcome that clinicians should consider when deciding to use an OCT device.

Structural and clinical changes in previously treated type 1 macular neovascularization in non-responder AMD eyes switched to brolucizumab.

PURPOSE: To examine structural and clinical changes in previously treated type 1 macular neovascularization (MNV) in non-responder age-related macular degeneration (nAMD) eyes switched to brolucizumab. Subretinal hyper-reflective material (SHRM), intraretinal (IRF) and subretinal fluid (SRF) presence, fibrovascular-pigment epithelium detachment (PED) height and central macular thickness (CMT) variation were analyzed using optical coherence tomography (OCT). METHODS: In this prospective study all patients underwent a complete ophthalmological evaluation including structural OCT at baseline (T0), one month (T1), three (T2), four (T3) and six months after switching to brolucizumab treatment (T4). Non-responder criterion was the persistence of IRF and SRF. Moreover, CMT and BCVA had shown worsening or no improvement before switching to brolucizumab. Clinical function and structural activity biomarkers were measured at each visit and changes were analyzed. P value <0.05 was considered statistically significant. RESULTS: Twenty eyes of twenty patients were enrolled. All the structural variables examined during the follow-up showed significant reductions. Decreases in IRF, SRF and PED were already significant at T1 (p < 0.05). SHRM was significantly reduced at T2 (p < 0.05). Structural biomarkers were absent at T3. At T4, all biomarkers remained stable while SHRM was no longer detectable in 18 patients. Changes in visual acuity from baseline to T4 were not significant. CONCLUSION: This short-term experience highlights that brolucizumab might be considered an effective treatment option in nAMD with type 1 MNV, as it can promote a reduction of structural activity biomarkers.

PREDICTIVE ROLE OF SWEPT SOURCE OCT ANGIOGRAPHY IN RELAPSING VOGT-KOYANAGI-HARADA DISEASE.

PURPOSE: The purpose of this study was to describe the optical coherence tomography (OCT) angiography findings as a predictive role in the chronic relapsing stage of Vogt-Koyanagi-Harada disease and its comparison with other imaging modalities such as fluorescein angiography, indocyanine-green angiography, and spectral domain OCT. METHODS: A 37-year-old woman from Bangladesh was diagnosed with Vogt-Koyanagi-Harada disease. She was evaluated 8 months before for a routine examination when she was in clinical remission. Full ophthalmic evaluation with multimodal imaging and OCT angiography was performed. RESULTS: Ophthalmic evaluation was unremarkable. Spectral domain OCT disclosed increased choroidal thickness in both eyes while swept source OCT angiography imaging showed choroidal flow voids well-matching hypofluorescent round lesions found by indocyanine-green angiography. A week later the disease reactivated. CONCLUSION: Optical coherence tomography angiography may provide novel insights into inflammatory activity of the choroid and potentially have a predictive role in relapsing Vogt-Koyanagi-Harada disease.

Hellp syndrome-related hypertensive chorioretinopathy: A multimodal imaging and optical coherence tomography angiography (OCTA) study.

INTRODUCTION: Pregnancy may be associated to unique retinal disorders and is associated to worsening of retinal disease that also occurs in non-pregnant females. We report a case of chorioretinopathy caused by pre-eclampsia associated to HELLP (Hemolysis-Elevated-Liver enzymes and Low Platelet count) syndrome. METHODS: Multimodal Imaging features of HELLP syndrome-related hypertensive chorioretinopathy including retinography, Spectral Domain-Optical Coherence Tomography (SD-OCT), Fluorescein angiography (FA), Indocyanine-green angiography (ICG) along with OCT-angiography (OCTA) are presented and discussed. RESULTS: Multimodal imaging and OCTA show both retinal and choroidal involvement by HELLP syndrome, resolved after hypertension treatment. CONCLUSIONS: Multimodal imaging is useful to study HELLP syndrome-related hypertensive chorioretinopathy. Moreover, OCTA is a new technology able to study and follow the circulatory status of the choriocapillaris during the disease.

Management of presumed candida endophthalmitis during the COVID-19 pandemic: Case report and review of the literature.

PURPOSE: To report the management of a case of candida endophthalmitis following intubation for SARS-CoV-2 ARDS, during the first lockdown, underlining consequently the limited access to the gold standard of care. METHODS: Case report. RESULTS: We have described the case of a 56-year-old Hispanic man who developed bilateral Candida endophthalmitis after one month of hospitalization in the intensive care unit for severe SARS-CoV-2 pneumonia. Multimodal imaging was obtained. Full serological screening for infection was done resulting negative. A diagnostic vitrectomy was impossible due to the limited access at the surgery room. Diagnosis of presumed candida endophthalmitis was made, according to a highly qualified center, and therapy with intravenous liposomal amphotericin B was administered. At three months, we observed lack of vitreous opacities and atrophic scars with no active lesions. CONCLUSIONS: Diagnosis of candida endophthalmitis was a challenge in this pandemic scenario, given the impossibility of performing a diagnostic vitrectomy. Therefore, it was mainly based on patient's clinical manifestations, multimodal imaging and on unanimity of collegiality between our hospital and the highly specialized center.

OCTA in macular intraretinal microvascular abnormalities: Retinal vascular density remodeling after panretinal photocoagulation.

PURPOSE: To describe a case of macular intraretinal microvascular abnormality (IRMA) detected with Optical Coherence Tomography Angiography (OCTA) and to show its remodeling and vascular density changes after panretinal photocoagulation (PRP) during an 18-month follow-up. METHODS: Case report. RESULTS: A 22-year-old female patient with proliferative diabetic retinopathy was found to have a small hyperreflective formation with posterior shadow cone and signal flow, located at the temporal margin of the fovea avascular zone (FAZ), identified as macular IRMA with OCTA. Her best-corrected-visual acuity was 20/20. Four months later the macular IRMA was larger and, in its context, there was also an increase in the flow area in B-scan. The patient underwent PRP and after 18 months we observed a regression of macular IRMA and an increase in the superficial capillary plexus vessel density in all sectors in comparison to baseline. CONCLUSION: OCTA is a non-invasive tool that recognize macular IRMA in diabetic retinopathy patient and it could be helpful to follow their qualitative and quantitative vascular evolution over time.

Real-life patient journey in neovascular age-related macular degeneration: a narrative medicine analysis in the Italian setting.

OBJECTIVES: To investigate the real-life experience of patients affected by neovascular age-related macular degeneration (nAMD), in the healthcare pathway for the management of the disease, using a "patient journey" and narrative method approach. METHODS: The patient journey of subjects affected by nAMD was designed using a process-mapping methodology involving a team from 11 Italian centres. Subsequently, narratives were collected from nAMD patients and family members. The interviews were analyzed using the narrative medicine methodology. RESULTS: Eleven specialized retina centres across Italy were involved and 205 narratives collected. In 29% of cases, patients underestimated their symptoms or attributed them to non-pathological causes, thus delaying the specialist consultation. The delay in accessing to care was due to a lack of awareness of this disease (50% of the participants didn't know what nAMD is) and to critical issues faced at first visit (long waiting lists, failed diagnosis, underestimation of the problem). Despite anti-VEGF therapies were perceived as effective in improving or stabilizing vision in 91% of narratives collected, 77% of patients still reduced or ceased daily activities such as reading and driving. Within the pathway of care there was not a multidisciplinary approach, and the patients were treated just by the ophthalmologist. CONCLUSIONS: nAMD may significantly affect the quality of life of affected patients, both from a functional and psychological point of view. The narrative medicine approach highlights some critical points in the healthcare journey of nAMD patients and represents a useful background in implementing patient management algorithms and pathways of care.

Diffuse posterior scleritis associated with HLA-B14* managed with multimodal imaging: case report and review of the literature.

PURPOSE: To report a case of posterior scleritis (PS) in an HLA-B*14 positive patient highlighting the crucial role of multimodal imaging and of autoimmune systemic disease screening for the diagnosis. METHODS: Case Report. RESULTS: A 73-year-old woman, complaining of ocular discomfort in right eye (RE), presented conjunctival hyperemia, keratic precipitates involving the anterior segment and four chorioretinal elevations with macular folds at fundus examination. Multimodal imaging and systemic diagnostic work-up were performed. While awaiting results, topical corticosteroid was prescribed. Four days later, worsening of exudative chorioretinal was detected. Except for erythrocyte sedimentation rate, tests were negative, while molecular typing of HLA genes was positive for HLA-B*14. Diagnosis of PS was confirmed by ultrasound examination while diagnosis of ankylosing spondylitis was made by rheumatologist. The patient was given systemic corticosteroid and one month later the posterior segment was unremarkable. CONCLUSION: HLA-B*14 may configure as a causal factor so autoimmune systemic disease screening should rule this out in management of PS, which should be diagnosed on the basis of multimodal imaging.

Macular hemorrhage after laser exposure and cannabinoid intake during a disco party.

PURPOSE: To describe two cases of macular hemorrhage in young patients, both occurred after laser exposure and cannabinoid intake during a disco party. OBSERVATIONS: Case 1: a 21-year-old man was evaluated at our Emergency Unit for sudden vision loss in the right eye (RE). Best-corrected visual acuity (BCVA) was count fingers in the RE and 20/20 in the left eye (LE). Fundus examination revealed a broad pre-retinal hemorrhage in macular region of RE, confirmed by optical coherence tomography. The patient reported vision loss, suddenly occurred after fixation of a laser source and cannabinoid intake during a disco party the night before. We administered a macular supplement and closely followed up the patient. After two months BCVA of the right eye was 20/20. CASE 2: The following day another 21-year-old man was referred to our Emergency Unit complaining of sudden vision loss in LE. As in Case 1, he reported to have fixed a laser beam as well as the consumption of cannabinoids at the same disco. BCVA was count fingers in the LE and 20/20 in the RE. Fundus examination showed a broad pre-retinal hemorrhage in macular region of LE. He had taken the macular supplement for two months and then the hemorrhage was reabsorbed. CONCLUSION AND IMPORTANCE: Laser exposure must be considered as a possible cause of macular hemorrhage. Furthermore, low cost of drugs and lack of formal control of laser sources may increase the emergence of new cases of retinal injuries especially among young people.

The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 (VA LV VFQ-48): Performance of the Italian version.

PURPOSE: The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 is among the most validated tools to collect patient-reported outcomes in a low-vision population. We have aimed to conduct a pilot validation of the Italian version of the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48. METHODS: The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 was translated using a standardized procedure and then administered to consecutive low-vision patients attending rehabilitation services in three centers. Patients were interviewed by a trained psychologist regarding the individual items of the tool. RESULTS: We included 131 patients with a mean visual acuity of 0.91 logMAR (standard deviation: 0.42 logMAR), mostly affected by age-related macular degeneration. The Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 showed high internal consistency (Cronbach's alpha: 0.98) and good item-test and item-rest correlation (median: 0.73 and 0.71, respectively). Both the overall score and the subscale (reading, visual motor, mobility and visual information) scores significantly correlated with visual acuity, reading acuity and speed. Reading speed achieved the best absolute correlation with the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 scores (Spearman r: 0.39-0.49). CONCLUSION: The Italian version of the Veterans Affairs Low-Vision Visual Functioning Questionnaire-48 is a valid tool to assess patients attending low-vision services. Revising a few items may further improve the tool.

Choroidal cleft simulating choroidal caverns in neovascular age-related macular degeneration.

The authors report a case of a female patient affected by neovascular age-related macular degeneration (AMD). In particular, multiple sub-retinal hyperreflective infiltrates were found on optical coherence tomography. Optical coherence tomography examination of her right eye displayed the presence of sub-retinal pigment epithelium hyporeflective spaces located beneath a hyperreflective fibrotic neovascularization. This case highlights the importance of differentiating choroidal clefts from choroidal caverns.

Vessel density, retinal thickness, and choriocapillaris vascular flow in myopic eyes on OCT angiography.

PURPOSE: To investigate foveal avascular zone area, macular vascular density, choroidal thickness, and outer retina and choriocapillaris flow in myopic eyes by OCT angiography. METHODS: Automated macular maps and flow calculations were retrospectively evaluated in 42 myopic and in 40 control eyes. RESULTS: Myopic eyes presented lower whole superficial vessel density (46.4 ± 4.9 vs. 51.6 ± 3.6%, P < 0.0001) and higher flow area in the outer retina (1.3 ± 0.2 vs. 1.1 ± 0.3 mm2, P = 0.0012). Between the myopic and non-myopic eyes, no significant differences could be detected in the choriocapillaris perfusion area (1.9 ± 0.07 vs. 1.9 ± 0.05 mm2, respectively; P = 0.55) and in the foveal avascular zone area (0.23 ± 0.1 vs. 0.26 ± 0.1 mm2, respectively; P = 0.12). The spherical correction positively correlated with superficial vessel density and negatively correlated with outer retina perfusion (P ≤ 0.0021). The superficial vessel density and the local retinal thickness positively correlated at all macular locations (P < 0.005), especially in the foveal region (P < 0.0001). CONCLUSIONS: Eyes with high myopia present reduced superficial vascular density and increased outer retina flow. Superficial vascular density and retinal thickness appear to be significantly correlated.

The volume of peripapillary vessels within the retinal nerve fibre layer: an optical coherence tomography angiography study of normal subjects.

BACKGROUND/AIMS: To investigate the contribution of vascular volume calculated by optical coherence tomography angiography (OCTA) to the measurement of peripapillary retinal nerve fibre layer (RNFL) thickness. METHODS: We used OCTA scans to build volumetric maps of the RNFL angiograms by thresholding the decorrelation images and summing the number of white pixels along the z-axis at each location. We used these maps to calculate the contribution of the vascular tissue to the RNFL thickness. RESULTS: We analysed 51 eyes from 36 subjects. The mean RNFL volume calculated on the peripapillary region was 0.607±0.045 mm3 and the mean vessel volume was 0.217±0.035 mm3, with a mean vessel/total RNFL ratio of 35.627%±3.942%. When evaluated in the peripapillary circular section, the total contribution of the vascular tissue to the global RNFL thickness was 29.071%±3.945%. The superior and inferior sectors showed the highest percentage of vascular tissue within the RNFL circular profile (31.369% and 34.788% respectively). CONCLUSIONS: We found that the vascular contribution to the RNFL thickness is 29.07±3.945%. This is much higher than what has been reported from calculations made on the structural OCT alone (13% reported by Hood et al and 11.3%±1.6% for the Cirrus OCT and 11.8%±1.4% for the Spectralis OCT reported by Patel et al). We conclude that evaluation of the vascular tissue contribution to the RNFL thickness with OCTA might be useful when performing precise quantification of the neuronal tissue.

Features of the normal choriocapillaris with OCT-angiography: Density estimation and textural properties.

The main objective of our work is to perform an in depth analysis of the structural features of normal choriocapillaris imaged with OCT Angiography. Specifically, we provide an optimal radius for a circular Region of Interest (ROI) to obtain a stable estimate of the subfoveal choriocapillaris density and characterize its textural properties using Markov Random Fields. On each binarized image of the choriocapillaris OCT Angiography we performed simulated measurements of the subfoveal choriocapillaris densities with circular Regions of Interest (ROIs) of different radii and with small random displacements from the center of the Foveal Avascular Zone (FAZ). We then calculated the variability of the density measure with different ROI radii. We then characterized the textural features of choriocapillaris binary images by estimating the parameters of an Ising model. For each image we calculated the Optimal Radius (OR) as the minimum ROI radius required to obtain a standard deviation in the simulation below 0.01. The density measured with the individual OR was 0.52 ± 0.07 (mean ± STD). Similar density values (0.51 ± 0.07) were obtained using a fixed ROI radius of 450 µm. The Ising model yielded two parameter estimates (ß = 0.34 ± 0.03; γ = 0.003 ± 0.012; mean ± STD), characterizing pixel clustering and white pixel density respectively. Using the estimated parameters to synthetize new random textures via simulation we obtained a good reproduction of the original choriocapillaris structural features and density. In conclusion, we developed an extensive characterization of the normal subfoveal choriocapillaris that might be used for flow analysis and applied to the investigation pathological alterations.

Transient Esotropia in the Child: Case Report and Review of the Literature.

The aim of this report is to investigate the possible causes of acute acquired onset of transient esotropia (AATE) in children and to help to differentiate ophthalmoplegic migraine (OM) from accommodative spasm (AS). A case of an 8-year-old Caucasian female affected by AATE and diplopia is described. The day before AATE onset, the patient complained of slight headache without nausea and vomiting, with spontaneous resolution. AATE diagnosis is challenging. The most likely ophthalmological causes of AATE are AS and OM. In these cases it is important to evaluate the presence of both a familial history of recurrent headaches and an AATE associated with migraine, ptosis, nausea, and vomiting. A full ophthalmological evaluation and a thorough refractive examination in cycloplegia are mandatory to exclude ophthalmological causes.

Intravitreal Anti-Vascular Endothelial Growth Factor Drugs for Retinal Angiomatous Proliferation in Real-Life Practice.

PURPOSE: To describe the outcomes of intravitreal anti-vascular endothelial growth factor (VEGF) in the treatment of retinal angiomatous proliferation (RAP) in real-life practice in 7 Italian centers under the Progetto Luce initiative. METHODS: Clinical data of 95 eyes of 95 patients affected by RAP, regularly followed up and treated with either intravitreal ranibizumab or bevacizumab over 12 months, were examined. After a loading phase of 3 consecutive injections, retreatments were administered following a pro-re-nata regimen on the basis of the persistence or the recurrence of subretinal/intraretinal fluid on optical coherence tomography, or leakage on fluorescein angiography. RESULTS: Overall, the mean best corrected visual acuity changed from 0.66 to 0.53 LogMAR (P: 0.0003); 36.8% of eyes gained at least 3 ETDRS lines, whereas 13.7% lost >3 lines at the end of the follow-up. Mean central retinal thickness improved from 384 µm at baseline to 262 µm at the 12-month examination (P < 0.001). A serous pigment epithelium detachment (PED) was identified in 68.4% of eyes at baseline, and it was still detectable in 30.5% at the end of follow-up. The mean number of injections was 4.4 over the follow-up. A significantly greater proportion of eyes showed PED resolution in the subgroup treated with ranibizumab (P < 0.001). CONCLUSIONS: Intravitreal anti-VEGF treatment in routine clinical practice allows a significant improvement in visual function in patients affected by RAP. A limited number of anti-VEGF injections are generally required in most cases.